Anesthetic considerations for the patient with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome)

Hereditary hemorrhagic telangiectasia (HHT), Osler-Weber-Rendu Syndrome, is an uncommon disease but may be present in many people who remain undiagnosed. It is an autosomal dominant disorder characterized by multiple arteriovenous malformations (AVMs) and telangiectases that affect multiple organ systems. Hereditary hemorrhagic telangiectasia patients have a propensity for bleeding, especially from the oropharynx, nasopharynx and gastrointestinal tract, as well as from rupture of AVMs of other organ systems. Anesthetic care of patients with HHT involves very specific interventions with regard to control of bleeding, maintaining adequate oxygenation, and balancing hemodynamic values to optimize perfusion without compromising anesthetic depth.